Primary localized cutaneous amyloidosis in patients with scleroderma.

Primary localized cutaneous amyloidosis (PLCA) is a relatively rare condition characterized by amyloid de-position exclusively in the dermis without involving the internal organs. Clinically, papular, macular and tumefac-tive forms are presented. Although PLCA may sometimes overlap with collagen vascular diseases, association with scleroderma is rare. We report here two cases of PLCA developing in patients with scleroderma. CASE REPORTS Case 1. A 70-year-old Japanese woman was referred to our department with swollen fingers on both hands. One year previously she had noticed pale, chilly and oedematous fingers and Raynaud's phenomenon. Physical examination revealed oedematous fingers (Fig. 1a), while skin sclerosis was not noted on either the dorsa of the hands or the forearms. Telangiectasia was found on her cheeks. Detailed physical examination revealed dotted pigmented papules on the upper back (Fig. 1b). Laboratory examination was positive for anti-nuclear antibody (1:2560, centromere pattern), but negative for antibodies against scleroderma-70 (Scl-70), ribonucleoprotein (RNP), SS-A and Jo-1. Skin biopsies were taken from the left middle finger, left lateral forearm and back. Histological features of specimens from the finger showed dermal sclerosis with thickened and homogenous collagen bundles in the mid-to-lower dermis (Fig. 1c), and those from the forearm showed a mild sclerosis with oedematous stage. Histological features of the specimen from the back showed amorphous deposits in the papillary dermis. Congo-red and Dylon staining showed positive findings in the papillary dermis (Fig. 1d). Case 2. A 62-year-old man was referred to our department with skin stiffness and pigmentation of both forearms. He stated that hyperpigmentation with itching had occurred one year previously, and that skin sclerosis had been present for more than 6 months. He was a builder and had been engaged in construction work, tiling roofs, and had used organic solvents containing silicon adhesives. Physical examination revealed that his fingers were pale and oedematous (Fig. 2a), and severe sclerosis with hyperpig-mentation was observed on his forearms, the dorsa of his hands, and his fingers. Neither large nor small pinching was possible. Furthermore, multiple hyperkeratotic whitish papules were localized on the lateral aspects of both forearms (Fig. 2b). Erythema and pigmentation was also seen on his abdomen and back, but without skin sclerosis. Laboratory examination for anti-nuclear antibody was positive (1:160, homogeneous and speckled), and anti-double-strand DNA antibody was 40.8 IU/ml (normal < 10 IU/ml). Results for antibodies against RNP, Scl-70, SS-A, and SS-B were all negative. Respiratory examination showed slight ventilation disorder, …